General Information About Childhood Extracranial Germ Cell Tumors
Stages of Childhood Extracranial Germ Cell Tumors
Recurrent Childhood Extracranial Germ Cell Tumors
Treatment Option Overview
Treatment Options for Childhood Extracranial Germ Cell Tumors
To Learn More About Childhood Cancer
Changes to This Summary (03/21/2012)
Get More Information From NCI
About PDQ
General Information About Childhood Extracranial Germ Cell Tumors
Childhood extracranial germ cell tumors form from developing sperm or egg cells that travel to parts of the body other than the brain.
As a fetus develops, certain cells form sperm in the testicles or eggs in the ovaries. Sometimes these cells travel to other parts of the body and grow into germ cell tumors. This summary is about germ cell tumors that form in parts of the body that are extracranial (outside the brain). Extracranial germ cell tumors are most common in teenagers 15 to 19 years old.
Childhood extracranial germ cell tumors may be benign or malignant.
Extracranial germ cell tumors may be benign (noncancer) or malignant (cancer).
There are three types of extracranial germ cell tumors.
Extracranial germ cell tumors are grouped into mature teratomas, immature teratomas, or malignant germ cell tumors:
Mature teratomas are the most common type of extracranial germ cell tumor. The cells of mature teratomas look very much like normal cells. Mature teratomas are benign and not likely to become cancer.
Immature teratomas have cells that look very different from normal cells. Immature teratomas are not cancer. They often contain several different types of tissue such as hair, muscle, and bone.
Malignant germ cell tumors are cancer. There are three types of malignant germ cell tumors:
- Yolk sac tumors: Tumors that make a hormone called alpha-fetoprotein (AFP).
- Germinomas: Tumors that make a hormone called beta-human chorionic gonadotropin (β-hCG).
- Choriocarcinomas: Tumors that make a hormone called beta-human chorionic gonadotropin (β-hCG).
Childhood extracranial germ cell tumors are grouped as gonadal or extragonadal.
Malignant extracranial germ cell tumors are grouped into gonadal and extragonadal.
Gonadal germ cell tumors form in the testicles or ovaries.
Testicular Germ Cell Tumors
Testicular germ cell tumors usually occur before the age of 4 years or in teenagers and young adults.
Testicular germ cell tumors in teenagers and young adults are different from those that form in early childhood. They are more like testicular cancer in adults. Testicular germ cell tumors are divided into two main types, seminoma and nonseminoma. (See the PDQ summary on Testicular Cancer Treatment 1 for more information.)
- Seminoma: These tumors make a hormone called beta-human chorionic gonadotropin (β-hCG).
- Nonseminoma: These tumors are usually large and cause symptoms. They tend to grow and spread more quickly than seminomas.
Boys older than 14 years with testicular germ cell tumors are treated in pediatric cancer centers, but the treatment is similar to that used in adults. (See the PDQ summary on Testicular Cancer Treatment 1 for more information.)
Ovarian Germ Cell Tumors
Ovarian germ cell tumors form in egg-making cells in an ovary. These tumors are more common in teenage girls and young women. Most ovarian germ cell tumors are benign teratomas. (See the PDQ summary on Ovarian Germ Cell Tumors Treatment 2 for more information.)
Extragonadal Extracranial Germ Cell Tumors
Extragonadal germ cell tumors form in areas other than the testicles or ovaries.
Most germ cell tumors that are not in the testicles, ovaries, or brain, form along the midline of the body. This includes the following:
- Sacrum (the large, triangle-shaped bone in the lower spine that forms part of the pelvis).
- Coccyx (the small bone at the bottom of the spine, also called the tailbone).
- Mediastinum (the area between the lungs).
- Back of the abdomen.
- Neck.
In younger children, extragonadal extracranial germ cell tumors usually occur at birth or in early childhood. Most of these tumors are teratomas in the sacrum or coccyx.
In older children, teenagers, and young adults, extragonadal extracranial germ cell tumors are often in the mediastinum.
The cause of most childhood extracranial germ cell tumors is unknown.
Having certain inherited disorders can increase the risk of developing an extracranial germ cell tumor.
Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. People who think they may be at risk should discuss this with their doctor. Possible risk factors for extracranial germ cell tumors include the following:
- Having certain genetic syndromes may increase the risk of developing childhood germ cell tumors:
- Klinefelter syndrome may increase the risk of developing germ cell tumors in the mediastinum.
- Swyer syndrome may increase the risk of developing germ cell tumors in the testes or ovaries.
- Having an undescended testicle may increase the risk of developing a testicular germ cell tumor.
Signs of childhood extracranial germ cell tumors depend on the type of tumor and where it is in the body.
Different tumors may cause the following signs and symptoms. Other conditions may cause these same symptoms. A doctor should be consulted if any of these problems occur.
- Most tumors of the sacrum and coccyx can be seen as a lump.
- A testicular tumor may cause a painless lump in the testicles.
- An ovarian germ cell tumor may cause:
- Pain or a lump in the abdomen.
- Fever.
- Constipation.
- No menstruation.
- Unusual vaginal bleeding.
Imaging studies and blood tests are used to detect (find) and diagnose childhood extracranial germ cell tumors.
The following tests and procedures may be used:
- Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. The testicles may be checked for lumps, swelling, or pain. A history of the patient's health habits and past illnesses and treatments will also be taken.
- Serum tumor marker test: A procedure in which a sample of blood is checked to measure the amounts of certain substances released into the blood by organs, tissues, or tumor cells in the body. Certain substances are linked to specific types of cancer when found in increased levels in the blood. These are called tumor markers.
Most malignant germ cell tumors release tumor markers. The following tumor markers are used to detect extracranial germ cell tumors:
- Alpha-fetoprotein (AFP).
- Beta-human chorionic gonadotropin (β-hCG).
- Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it.
- Cytogenetic analysis: A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes.
- Immunohistochemistry study: A laboratory test in which a substance such as an antibody, dye, or radioisotope is added to a sample of cancer tissue to test for certain antigens. This type of study is used to tell the difference between different types of cancer.
- Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
- CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
- Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later.
- Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. In some cases, the tumor is removed during surgery and then a biopsy is done.
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on the following:
- The type of germ cell tumor.
- Where the tumor first began to grow.
- The stage of the cancer (whether it has spread to nearby areas or to other places in the body).
- Whether the tumor can be completely removed by surgery.
- The patient's age and general health.
- Whether the cancer has just been diagnosed or has recurred (come back).
The prognosis for childhood extracranial germ cell tumors, especially ovarian germ cell tumors, is good.
Stages of Childhood Extracranial Germ Cell Tumors
After a childhood extracranial germ cell tumor has been diagnosed, tests are done to find out if cancer cells have spread from where the tumor started to nearby areas or to other parts of the body.
The process used to find out if cancer has spread from where the tumor started to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. In some cases, staging may follow surgery to remove the tumor.
The following procedures may be used:
- MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging.
- Bone scan: A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner.
- Thoracentesis: The removal of fluid from the space between the lining of the chest and the lung, using a needle. A pathologist views the fluid under a microscope to look for cancer cells.
- Paracentesis: The removal of fluid from the space between the lining of the abdomen and the organs in the abdomen, using a needle. A pathologist views the fluid under a microscope to look for cancer cells.
The results from tests and procedures used to detect and diagnose childhood extracranial germ cell tumor may also be used in staging.
There are three ways that cancer spreads in the body.
The three ways that cancer spreads in the body are:
- Through tissue. Cancer invades the surrounding normal tissue.
- Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
- Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.
When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.
The following stages are commonly used for childhood nonseminoma testicular germ cell tumors:
In stage I, the cancer is found only in the testicle and is completely removed by surgery. Tumor marker levels return to normal after surgery.
In stage II, the cancer is removed by surgery and some cancer cells remain in the scrotum or cancer that can only be seen with a microscope has spread to the scrotum or spermatic cord. Tumor marker levels do not return to normal after surgery and may increase.
In stage III, the cancer has spread to one or more lymph nodes in the abdomen and is not completely removed by surgery. The cancer that remains after surgery can be seen without a microscope.
In stage IV, the cancer has spread to distant parts of the body such as the liver.
The following stages may be used for childhood ovarian germ cell tumors:
In stage I, the cancer is in the ovary and can be completely removed by surgery.
In stage II, one of the following is true:
- The cancer is not completely removed by surgery. The remaining cancer can be seen with a microscope only.
- The cancer has spread to the lymph nodes and can be seen with a microscope only.
- The cancer has spread to the capsule (outer covering) of the ovary.
In stage III, one of the following is true:
- The cancer is not completely removed by surgery. The remaining cancer can be seen without a microscope.
- The cancer has spread to lymph nodes and the lymph nodes are 2 centimeters or larger.
- The cancer is found in fluid in the abdomen.
In stage IV, the cancer has spread to the lung, liver, brain, or bone.
Another staging system which may be used for childhood ovarian germ cell tumors is as follows:
In stage I, cancer is found in one or both of the ovaries and has not spread. Stage I is divided into stage IA, stage IB, and stage IC.
- Stage IA: Cancer is found in a single ovary.
- Stage IB: Cancer is found in both ovaries.
- Stage IC: Cancer is found in one or both ovaries and one of the following is true:
- cancer is found on the outside surface of one or both ovaries; or
- the capsule (outer covering) of the tumor has ruptured (broken open); or
- cancer cells are found in fluid that has collected in the abdomen; or
- cancer cells are found in washings of the peritoneal cavity (the body cavity that contains most of the organs in the abdomen).
In stage II, cancer is found in one or both ovaries and has spread into other areas of the pelvis. Stage II is divided into stage IIA, stage IIB, and stage IIC.
- Stage IIA: Cancer has spread to the uterus and/or the fallopian tubes (the long slender tubes through which eggs pass from the ovaries to the uterus).
- Stage IIB: Cancer has spread to other tissue within the pelvis such as the bladder, rectum, or vagina.
- Stage IIC: Cancer has spread to the uterus and/or fallopian tubes and/or other tissue within the pelvis and one of the following is true:
- cancer is found on the outside surface of one or both ovaries; or
- the capsule (outer covering) of the tumor has ruptured (broken open); or
- cancer cells are found in fluid that has collected in the abdomen; or
- cancer cells are found in washings of the peritoneal cavity (the body cavity that contains most of the organs in the abdomen).
In stage III, cancer is found in one or both ovaries and has spread to other parts of the abdomen. Stage III is divided into stage IIIA, stage IIIB, and stage IIIC as follows:
- Stage IIIA: The tumor is found only in the pelvis, but cancer cells that only can be seen with a microscope have spread to the surface of the peritoneum (tissue that lines the abdominal wall and covers most of the organs in the abdomen) or to the small bowel.
- Stage IIIB: Cancer has spread to the peritoneum but is 2 centimeters or smaller in diameter.
- Stage IIIC: Cancer has spread to the peritoneum and is larger than 2 centimeters in diameter and/or has spread to lymph nodes in the abdomen.
Cancer that has spread to the surface of the liver is also considered stage III disease.
In stage IV, cancer is found in one or both ovaries and has metastasized (spread) beyond the abdomen to other parts of the body.
Cancer that has spread to tissues in the liver is also considered stage IV disease.
The following stages are commonly used for extragonadal extracranial germ cell tumors:
In stage I, the cancer is in one place and can be completely removed by surgery. For tumors at the base of the tailbone, the cancer and tailbone are completed removed by surgery. Tumor marker levels return to normal after surgery.
In stage II, the cancer has spread to nearby tissues and/or lymph nodes and is not completely removed by surgery. The cancer remaining after surgery can be seen with a microscope only. Tumor marker levels do not return to normal after surgery and may increase.
In stage III, one of the following is true:
- The cancer is not completely removed by surgery. The cancer remaining after surgery can be seen without a microscope.
- The cancer has spread to lymph nodes and is larger than 2 centimeters in diameter.
In stage IV, the cancer has spread to distant parts of the body, including the liver.
Recurrent Childhood Extracranial Germ Cell Tumors
Recurrent childhood extracranial germ cell tumor is cancer that has recurred (come back) after it has been treated. The cancer may come back in the same place or in other parts of the body.
The number of patients who have tumors that come back is small. Most recurrent germ cell tumors occur within three years of surgery. About half of the teratomas that recur in the sacrum or coccyx are malignant, so follow-up is important.
Treatment Option Overview
There are different types of treatment for children with extracranial germ cell tumors.
Different types of treatments are available for children with extracranial germ cell tumors. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.
Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.
Children with extracranial germ cell tumors should have their treatment planned by a team of health care providers who are experts in treating cancer in children.
Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with extracranial germ cell tumors and who specialize in certain areas of medicine. These may include the following specialists:
- Pediatric surgeon.
- Pediatric hematologist.
- Radiation oncologist.
- Endocrinologist.
- Pediatric nurse specialist.
- Rehabilitation specialist.
- Psychologist.
- Social worker.
- Geneticist.
Some cancer treatments cause side effects months or years after treatment has ended.
Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following:
- Physical problems.
- Changes in mood, feelings, thinking, learning, or memory.
- Second cancers (new types of cancer).
For example, late effects of surgery to remove tumors in the sacrum or coccyx include constipation, loss of bowel and bladder control, and scars.
Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer 4 for more information).
Three types of standard treatment are used:
Surgery to completely remove the tumor is done whenever possible. If the tumor is very large, chemotherapy may be given first, to make the tumor smaller and decrease the amount of tissue that needs to be removed during surgery. The following types of surgery may be used:
- Resection: Surgery to remove tissue or part or all of an organ. If cancer is in the coccyx, the entire coccyx is removed.
- Tumor debulking: A surgical procedure in which as much of the tumor as possible is removed. Some tumors may not be able to be completely removed.
- Radical inguinal orchiectomy: Surgery to remove one or both testicles through an incision (cut) in the groin.
- Unilateral salpingo-oophorectomy: Surgery to remove one ovary and one fallopian tube.
Watchful waiting is closely monitoring a patient’s condition without giving any treatment until symptoms appear or change. For childhood extracranial germ cell tumors, this includes physical exams, imaging tests, and tumor marker tests.
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the type and stage of the cancer being treated.
New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI Web site 5.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.
Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
For childhood extracranial germ cell tumors, alpha-fetoprotein (AFP) tests are done to see if treatment is working. Continued high levels of AFP may mean the cancer is still growing. For at least 3 years after surgery, follow-up will include regular physical exams, imaging tests, and tumor marker tests.
Treatment Options for Childhood Extracranial Germ Cell Tumors
A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.
Mature and Immature TeratomasTreatment of mature and immature teratomas in the sacrum or coccyx is usually surgery followed by watchful waiting. Most teratomas can be removed completely. If the tumor is in the coccyx, the entire coccyx is removed. Chemotherapy may be given if the tumor comes back.
Treatment of mature and immature teratomas that are not in the sacrum or coccyx is usually surgery followed by watchful waiting. A second surgery may be done to remove any remaining cancer.
Sometimes a mature or immature teratoma also has malignant cells. The teratoma and malignant cells may need to be treated differently. The best treatment for the malignant cells is not known.
Regular follow-up exams with imaging tests and the alpha-fetoprotein (AFP) tumor marker test will be done for at least 3 years.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with childhood teratoma 6. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 7.
Malignant Gonadal Germ Cell TumorsMalignant Testicular Germ Cell Tumors
Treatment of malignant testicular germ cell tumors may include the following:
For boys younger than 15 years:
- Surgery (radical inguinal orchiectomy) followed by watchful waiting for stage I tumors. Chemotherapy may be given if the tumor comes back.
- Surgery (radical inguinal orchiectomy) followed by combination chemotherapy for stage II-IV tumors.
- A clinical trial of fewer cycles of chemotherapy after surgery.
For boys 15 years and older:
Malignant testicular germ cell tumors in boys 15 years and older are treated differently than they are in young boys. Surgery may include removal of lymph nodes in the abdomen. Treatment depends on whether the tumor is a seminoma or a nonseminoma. (See the PDQ summary on Testicular Cancer Treatment 1 for more information.)
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with childhood malignant testicular germ cell tumor 8. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 7.
Malignant Ovarian Germ Cell TumorsTreatment of childhood malignant ovarian germ cell tumors in young girls may include the following:
- Surgery (unilateral salpingo-oophorectomy) followed by watchful waiting for stage I dysgerminoma or immature teratoma tumors. Chemotherapy may be given if the tumor comes back.
- Surgery (unilateral salpingo-oophorectomy) followed by combination chemotherapy for stages II-IV tumors.
- Chemotherapy to make the tumor smaller, followed by surgery (unilateral salpingo-oophorectomy).
- Surgery (tumor debulking) followed by chemotherapy.
- A clinical trial of fewer cycles of chemotherapy after surgery.
- A clinical trial of surgery (unilateral salpingo-oophorectomy) followed by watchful waiting for stage I tumors that are not a dysgerminoma or immature teratoma.
The treatment for adolescents and young adults with ovarian germ cell tumor is similar to the treatment for adults. (See the PDQ treatment summary on Ovarian Germ Cell Tumors 2 for more information.)
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with childhood malignant ovarian germ cell tumor 9. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 7.
Malignant Extragonadal Germ Cell TumorsTreatment of childhood extragonadal malignant germ cell tumors may include the following:
- Surgery to remove tumors in the mediastinum (the area between the lungs).
- Surgery followed by combination chemotherapy.
- Combination chemotherapy followed by surgery.
- Chemotherapy only.
- A clinical trial of a new chemotherapy regimen.
(See the PDQ treatment summary on Extragonadal Germ Cell Tumors 10 for more information.)
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with childhood extragonadal germ cell tumor 11. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 7.
Recurrent Childhood Malignant Extracranial Germ Cell TumorsThere is no standard treatment for recurrent childhood malignant extracranial germ cell tumors. Treatment is usually within in a clinical trial and may include the following:
- Surgery followed by combination chemotherapy, for malignant testicular germ cell tumors that recur after being treated with surgery and watchful waiting.
- Surgery for tumors that come back in the sacrum or coccyx. Chemotherapy may be given before surgery, to shrink the tumor. If any of the tumor remains after surgery, radiation therapy may be added.
- Surgery followed by chemotherapy, for malignant ovarian germ cell tumors that recur after being treated with surgery and watchful waiting.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent childhood malignant germ cell tumor 12. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 7.
To Learn More About Childhood Cancer
For more information from the National Cancer Institute about childhood extracranial germ cell tumors, see the Extracranial Germ Cell Tumor (Childhood) Home Page 13.
For more childhood cancer information and other general cancer resources from the National Cancer Institute, see the following:
- What You Need to Know About™ Cancer 14
- Childhood Cancers 15
- CureSearch for Children's Cancer 16
- Late Effects of Treatment for Childhood Cancer 4
- Adolescents and Young Adults with Cancer 17
- Young People with Cancer: A Handbook for Parents 18
- Care for Children and Adolescents with Cancer 19
- Understanding Cancer Series: Cancer 20
- Cancer Staging 21
- Coping with Cancer: Supportive and Palliative Care 22
- Questions to Ask Your Doctor About Cancer 23
- Cancer Library 24
- Information for Survivors/Caregivers/Advocates 25
Changes to This Summary (03/21/2012)
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Changes were made to this summary to match those made to the health professional version.
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About PDQ
PDQ is a comprehensive cancer database available on NCI's Web site.
PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at NCI's Web site 27. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.
PDQ contains cancer information summaries.
The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries are available in two versions. The health professional versions provide detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions provide current and accurate cancer information.
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Editorial Boards made up of experts in oncology and related specialties are responsible for writing and maintaining the cancer information summaries. The summaries are reviewed regularly and changes are made as new information becomes available. The date on each summary ("Date Last Modified") indicates the time of the most recent change.
PDQ also contains information on clinical trials.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." In the United States, about two-thirds of children with cancer are treated in a clinical trial at some point in their illness.
Listings of clinical trials are included in PDQ and are available online at NCI's Web site 7. Descriptions of the trials are available in health professional and patient versions. For additional help in locating a childhood cancer clinical trial, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).
The PDQ database contains listings of groups specializing in clinical trials.
The Children's Oncology Group (COG) is the major group that organizes clinical trials for childhood cancers in the United States. Information about contacting COG is available on the NCI Web site 27 or from the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).
Glossary Termsabdomen (AB-doh-men)The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.alpha-fetoprotein (AL-fuh-FEE-toh-PROH-teen) A protein normally produced by a fetus. AFP levels are usually undetectable in the blood of healthy adult men or women (who are not pregnant). An elevated level of AFP suggests the presence of either a primary liver cancer or germ cell tumor. Also called AFP.antibody (AN-tee-BAH-dee) A protein made by plasma cells (a type of white blood cell) in response to an antigen (a substance that causes the body to make a specific immune response). Each antibody can bind to only one specific antigen. The purpose of this binding is to help destroy the antigen. Some antibodies destroy antigens directly. Others make it easier for white blood cells to destroy the antigen.antigen (AN-tih-jen) Any substance that causes the body to make a specific immune response.benign (beh-NINE) Not cancerous. Benign tumors may grow larger but do not spread to other parts of the body. Also called nonmalignant.beta-human chorionic gonadotropin (BAY-tuh-HYOO-mun KOR-ee-AH-nik goh-NA-doh-TROH-pin) A hormone found in the blood and urine during pregnancy. It may also be found in higher than normal amounts in patients with some types of cancer, including testicular, ovarian, liver, stomach, and lung cancers, and in other disorders. Measuring the amount of beta-human chorionic gonadotropin in the blood or urine of cancer patients may help to diagnose cancer and find out how well cancer treatment is working. Beta-human chorionic gonadotropin is a type of tumor marker. Also called beta-hCG.biopsy (BY-op-see) The removal of cells or tissues for examination by a pathologist. The pathologist may study the tissue under a microscope or perform other tests on the cells or tissue. There are many different types of biopsy procedures. The most common types include: (1) incisional biopsy, in which only a sample of tissue is removed; (2) excisional biopsy, in which an entire lump or suspicious area is removed; and (3) needle biopsy, in which a sample of tissue or fluid is removed with a needle. When a wide needle is used, the procedure is called a core biopsy. When a thin needle is used, the procedure is called a fine-needle aspiration biopsy.blood (blud) A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.blood chemistry study (blud KEH-mih-stree STUH-dee) A procedure in which a sample of blood is examined to measure the amounts of certain substances made in the body. An abnormal amount of a substance can be a sign of disease in the organ or tissue that produces it.cancer (KAN-ser) A term for diseases in which abnormal cells divide without control and can invade nearby tissues. Cancer cells can also spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is a cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is a cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is a cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord. Also called malignancy.cell (sel) The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.chest x-ray (chest EX-ray) An x-ray of the structures inside the chest. An x-ray is a type of high-energy radiation that can go through the body and onto film, making pictures of areas inside the chest, which can be used to diagnose disease.choriocarcinoma (KOR-ee-oh-KAR-sih-NOH-muh) A malignant, fast-growing tumor that develops from trophoblastic cells (cells that help an embryo attach to the uterus and help form the placenta). Almost all choriocarcinomas form in the uterus after fertilization of an egg by a sperm, but a small number form in a testis or an ovary. Choriocarcinomas spread through the blood to other organs, especially the lungs. They are a type of gestational trophoblastic disease. Also called chorioblastoma, chorioepithelioma, and chorionic carcinoma.chromosome (KROH-muh-some) Part of a cell that contains genetic information. Except for sperm and eggs, all human cells contain 46 chromosomes.clinical trial (KLIH-nih-kul TRY-ul) A type of research study that tests how well new medical approaches work in people. These studies test new methods of screening, prevention, diagnosis, or treatment of a disease. Also called clinical study.coccyx (KOK-six) The small bone at the bottom of the spine. It is made up of 3-5 fused bones. Also called tailbone.condition (kun-DIH-shun) In medicine, a health problem with certain characteristics or symptoms.constipation (KON-stih-PAY-shun) A condition in which stool becomes hard, dry, and difficult to pass, and bowel movements don’t happen very often. Other symptoms may include painful bowel movements, and feeling bloated, uncomfortable, and sluggish.contrast material (KON-trast muh-TEER-ee-ul) A dye or other substance that helps show abnormal areas inside the body. It is given by injection into a vein, by enema, or by mouth. Contrast material may be used with x-rays, CT scans, MRI, or other imaging tests.CT scan (… skan) A series of detailed pictures of areas inside the body taken from different angles. The pictures are created by a computer linked to an x-ray machine. Also called CAT scan, computed tomography scan, computerized axial tomography scan, and computerized tomography.cytogenetics (SY-toh-jeh-NEH-tix) The study of chromosomes and chromosomal abnormalities.diagnosis (DY-ug-NOH-sis) The process of identifying a disease, such as cancer, from its signs and symptoms.extracranial (EK-struh-KRAY-nee-ul) Outside of the cranium (bones that surround the brain).extracranial germ cell tumor (EK-struh-KRAY-nee-ul jerm sel TOO-mer) A rare cancer that forms in germ cells in the testicle or ovary, or in germ cells that have traveled to areas of the body other than the brain (such as the chest, abdomen, or tailbone). Germ cells are reproductive cells that develop into sperm in males and eggs in females.extragonadal (EK-struh-goh-NA-dul) An area of the body other than the ovaries or testes.extragonadal germ cell tumor (EK-struh-goh-NA-dul jerm sel TOO-mer) A rare cancer that develops in germ cells that are found in areas of the body other than the ovary or testicle (such as the brain, chest, abdomen, or tailbone). Germ cells are reproductive cells that develop into sperm in males and eggs in females.fetus (FEE-tus) In humans, an unborn baby that develops and grows inside the uterus (womb). The fetal period begins 8 weeks after fertilization of an egg by a sperm and ends at the time of birth.fever (FEE-ver) An increase in body temperature above normal (98.6 degrees F), usually caused by disease.genetic (jeh-NEH-tik) Inherited; having to do with information that is passed from parents to offspring through genes in sperm and egg cells.germ cell tumor (jerm sel TOO-mer) A type of tumor that begins in the cells that give rise to sperm or eggs. Germ cell tumors can occur almost anywhere in the body and can be either benign or malignant.gonad (GOH-nad) The part of the reproductive system that produces and releases eggs (ovary) or sperm (testicle/testis).hormone (HOR-mone) One of many chemicals made by glands in the body. Hormones circulate in the bloodstream and control the actions of certain cells or organs. Some hormones can also be made in the laboratory.immature teratoma (IH-muh-CHOOR TAYR-uh-TOH-muh) A rare type of germ cell tumor (type of tumor that begins in the cells that give rise to sperm or eggs). Immature teratomas often contain several different types of tissue such as hair, muscle, and bone.immunohistochemistry (IH-myoo-noh-HIS-toh-KEH-mih-stree) A technique used to identify specific molecules in different kinds of tissue. The tissue is treated with antibodies that bind the specific molecule. These are made visible under a microscope by using a color reaction, a radioisotope, colloidal gold, or a fluorescent dye. Immunohistochemistry is used to help diagnose diseases, such as cancer, and to detect the presence of microorganisms. It is also used in basic research to understand how cells grow and differentiate (become more specialized).injection (in-JEK-shun) Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."Klinefelter syndrome (KLINE-fel-ter SIN-drome) A genetic disorder in males caused by having one or more extra X chromosomes. Males with this disorder may have larger than normal breasts, a lack of facial and body hair, a rounded body type, and small testicles. They may learn to speak much later than other children and may have difficulty learning to read and write. Klinefelter syndrome increases the risk of developing extragonadal germ cell tumors and breast cancer.laboratory test (LA-bruh-tor-ee...) A medical procedure that involves testing a sample of blood, urine, or other substance from the body. Tests can help determine a diagnosis, plan treatment, check to see if treatment is working, or monitor the disease over time.lung (lung) One of a pair of organs in the chest that supplies the body with oxygen, and removes carbon dioxide from the body.malignant (muh-LIG-nunt) Cancerous. Malignant cells can invade and destroy nearby tissue and spread to other parts of the body.mature teratoma (muh-CHOOR TAYR-uh-TOH-muh) A type of benign (not cancer) germ cell tumor (type of tumor that begins in the cells that give rise to sperm or eggs) that often contains several different types of tissue such as hair, muscle, and bone. Also called dermoid cyst.mediastinum (MEE-dee-uh-STY-num) The area between the lungs. The organs in this area include the heart and its large blood vessels, the trachea, the esophagus, the thymus, and lymph nodes but not the lungs.medical history (MEH-dih-kul HIH-stuh-ree) A record of information about a person’s health. A personal medical history may include information about allergies, illnesses, surgeries, immunizations, and results of physical exams and tests. It may also include information about medicines taken and health habits, such as diet and exercise. A family medical history includes health information about a person’s close family members (parents, grandparents, children, brothers, and sisters). This includes their current and past illnesses. A family medical history may show a pattern of certain diseases in a family.menstruation (MEN-stroo-WAY-shun) Periodic discharge of blood and tissue from the uterus. From puberty until menopause, menstruation occurs about every 28 days when a woman is not pregnant.microscope (MY-kroh-SKOPE) An instrument that is used to look at cells and other small objects that cannot be seen with the eye alone.nonseminoma (NON-seh-mih-NOH-muh) A type of cancer that begins in cells that form sperm or eggs. There are several types of nonseminoma tumors, including embryonal carcinoma, malignant teratoma, choriocarcinoma, and yolk sac tumor. These tumors are usually made up of more than one type of cancer cell. Although nonseminomas occur most often in the testicles or ovaries, they can occur in other tissues, such as the brain, chest, or abdomen. This happens when cells that have the ability to form sperm or eggs are found in other parts of the body.organ (OR-gun) A part of the body that performs a specific function. For example, the heart is an organ.ovarian germ cell tumor (oh-VAYR-ee-un jerm sel TOO-mer) An abnormal mass of tissue that forms in germ (egg) cells in the ovary (female reproductive gland in which the eggs are formed). These tumors usually occur in teenage girls or young women, usually affect just one ovary, and can be benign (not cancer) or malignant (cancer). The most common ovarian germ cell tumor is called dysgerminoma.ovary (OH-vuh-ree) One of a pair of female reproductive glands in which the ova, or eggs, are formed. The ovaries are located in the pelvis, one on each side of the uterus.pathologist (puh-THAH-loh-jist) A doctor who identifies diseases by studying cells and tissues under a microscope.PDQ PDQ is an online database developed and maintained by the National Cancer Institute. Designed to make the most current, credible, and accurate cancer information available to health professionals and the public, PDQ contains peer-reviewed summaries on cancer treatment, screening, prevention, genetics, complementary and alternative medicine, and supportive care; a registry of cancer clinical trials from around the world; and directories of physicians, professionals who provide genetics services, and organizations that provide cancer care. Most of this information, and more specific information about PDQ, can be found on the NCI's Web site at http://www.cancer.gov/cancertopics/pdq. Also called Physician Data Query.pediatric (pee-dee-A-trik) Having to do with children.pelvis (PEL-vus) The lower part of the abdomen, located between the hip bones.physical examination (FIH-zih-kul eg-ZA-mih-NAY-shun) An exam of the body to check for general signs of disease.prognosis (prog-NO-sis) The likely outcome or course of a disease; the chance of recovery or recurrence.radioisotope (RAY-dee-oh-I-suh-tope) An unstable form of a chemical element that releases radiation as it breaks down and becomes more stable. Radioisotopes may occur in nature or be made in a laboratory. In medicine, they are used in imaging tests and in treatment. Also called radionuclide.recover (ree-KUH-ver) To become well and healthy again.recur (ree-KER) To come back or to return.recurrent cancer (ree-KER-ent KAN-ser) Cancer that has recurred (come back), usually after a period of time during which the cancer could not be detected. The cancer may come back to the same place as the original (primary) tumor or to another place in the body. Also called recurrence.risk factor (... FAK-ter) Something that increases the chance of developing a disease. Some examples of risk factors for cancer are age, a family history of certain cancers, use of tobacco products, being exposed to radiation or certain chemicals, infection with certain viruses or bacteria, and certain genetic changes.sacrum (SAY-krum) The large, triangle-shaped bone in the lower spine that forms part of the pelvis. It is made of 5 fused bones of the spine.seminoma (SEH-mih-NOH-muh) A type of cancer that begins in cells that make sperm or eggs. Seminomas occur most often in the testicles or the ovaries. They may also occur in other organs, such as the brain, chest, or abdomen. This happens when cells that have the ability to form sperm or eggs are found in other parts of the body. Seminomas grow and spread slowly.serum tumor marker test (SEER-um TOO-mer MAR-ker ...) A blood test that measures the amount of substances called tumor markers (or biomarkers). Tumor markers are released into the blood by tumor cells or by other cells in response to tumor cells. A high level of a tumor marker may be a sign of cancer.sonogram (SAH-noh-gram) A computer picture of areas inside the body created by bouncing high-energy sound waves (ultrasound) off internal tissues or organs. Also called ultrasonogram.sperm (spurm) The male reproductive cell, formed in the testicle. A sperm unites with an egg to form an embryo.spine (spine) The bones, muscles, tendons, and other tissues that reach from the base of the skull to the tailbone. The spine encloses the spinal cord and the fluid surrounding the spinal cord. Also called backbone, spinal column, and vertebral column.stage (stayj) The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.surgery (SER-juh-ree) A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.symptom (SIMP-tum) An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.syndrome (SIN-drome) A set of symptoms or conditions that occur together and suggest the presence of a certain disease or an increased chance of developing the disease.tailbone (TAYL-bone) The small bone at the bottom of the spine. It is made up of 3-5 fused bones. Also called coccyx.teratoma (TAYR-uh-TOH-muh) A type of germ cell tumor that may contain several different types of tissue, such as hair, muscle, and bone. Teratomas occur most often in the ovaries in women, the testicles in men, and the tailbone in children. Not all teratomas are malignant.testicle (TES-tih-kul) One of two egg-shaped glands inside the scrotum that produce sperm and male hormones. Also called testis.testicular cancer (tes-TIH-kyuh-ler KAN-ser) Cancer that forms in tissues of one or both testicles. Testicular cancer is most common in young or middle-aged men. Most testicular cancers begin in germ cells (cells that make sperm) and are called testicular germ cell tumors.testicular germ cell tumor (tes-TIH-kyuh-ler jerm sel TOO-mer) A type of tumor that forms in a testicle from germ cells (cells that make sperm). Two main types of testicular germ cell tumors are seminomas and nonseminomas. Seminomas grow and spread slowly and are sensitive to radiation therapy. Nonseminomas grow and spread more quickly than seminomas. There are several different types of nonseminomas.tissue (TIH-shoo) A group or layer of cells that work together to perform a specific function.tumor (TOO-mer) An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancer), or malignant (cancer). Also called neoplasm.tumor marker (TOO-mer MAR-ker) A substance found in tissue, blood, or other body fluids that may be a sign of cancer or certain benign (noncancerous) conditions. Most tumor markers are made by both normal cells and cancer cells, but they are made in larger amounts by cancer cells. A tumor marker may help to diagnose cancer, plan treatment, or find out how well treatment is working or if cancer has come back. Examples of tumor markers include CA-125 (in ovarian cancer), CA 15-3 (in breast cancer), CEA (in colon cancer), and PSA (in prostate cancer).ultrasound (UL-truh-SOWND) A procedure in which high-energy sound waves are bounced off internal tissues or organs and make echoes. The echo patterns are shown on the screen of an ultrasound machine, forming a picture of body tissues called a sonogram. Also called ultrasonography.undescended testicles (UN-deh-SEN-ded TES-tih-kuls) A condition in which one or both testicles fail to move from the abdomen, where they develop before birth, into the scrotum. Undescended testicles may increase the risk for development of testicular cancer. Also called cryptorchidism.vaginal (VA-jih-nul) Having to do with the vagina (the birth canal).vein (vayn) A blood vessel that carries blood to the heart from tissues and organs in the body.x-ray (EX-ray) A type of radiation used in the diagnosis and treatment of cancer and other diseases. In low doses, x-rays are used to diagnose diseases by making pictures of the inside of the body. In high doses, x-rays are used to treat cancer. |

